Pharyngocutaneous Fistula in Salvage Laryngectomy: Use of the Stapler Versus Manual Suturing Technique.

A pharyngocutaneous fistula (PCF) is the most common complication after salvage total laryngectomy (STL) with an incidence ranging from 5 to 73%, causing an increase in morbidity and delaying the patient's recovery. A surgical technique to prevent PCF formation is the use of the stapler to suture the pharyngeal mucosa.  We have reviewed the medical files of 91 patients who had undergone STL, dividing the patients into two groups (manual suture vs. stapler), based on the type of pharyngeal suture performed during the STL. We found 12/49 (24.5%) cases of a PCF in group A (manual) and 5/42 (11.9%) cases of a PCF in group B (stapler). There is a statistically significant difference between the two groups with the patients in group B presenting better results in terms of the reduction of the surgical time, the length of the hospital stay and also in the restarting of oral feeding. The advantages of mechanical suturing with the stapler are reduced surgical times, a lower PCF formation rate, which involves an early removal of the nasogastric tube, and reduced hospitalization times. Further studies with a larger case series will be necessary to confirm the results obtained and to standardize the surgical technique.

Diffuse Idiopathic Skeletal Hyperostosis (DISH): Role of Logopedic Rehabilitation in Dysphagia.

Forestier's disease is a systemic, degenerative metabolic condition, which is still insufficiently investigated and understood, characterized by the progressive ossification of the ligaments and entheses. This case describes a 63-year-old man admitted to our department after several years of failed attempts to obtain a definitive diagnosis presenting with a painless mass in the pre-auricular region, gradually worsening dysphonia, severe dysphagia relating to solids, stiffness, and mild pain at the back of the neck. After performing further diagnostic tests, in addition to a pleomorphic adenoma, the simultaneous presence of diffuse spondylarthrosis on the cervical spine was highlighted, with beak-like osteophytes at C2-C5 resulting in esophageal compression. Given the absence of upper digestive endoscopy abnormalities, we initiated an intense logopedic and postural rehabilitative treatment, which led to a significant improvement in the patient's dysphagia symptoms. In addition, we limited the use of medical therapy to solely indomethacin in order to control the osteophytic process.

Small-Cell Carcinoma of Nasopharynx: A Case Report of Unusual Localization.

Neuroendocrine tumors are a spectrum of rare and highly heterogeneous neoplasms with distinct functional and biological behavior in relation to location, tumor size, and histological differentiation. Neuroendocrine tumors arise from the neuroendocrine cells of the diffuse neuroendocrine system located in almost every organ. Neuroendocrine tumors in the head and neck district are usually reported in sinonasal cavities and larynx. We present the case of a nasopharyngeal small-cell neuroendocrine carcinoma, which, as far as we know, is the 16th case reported in literature.

Supracricoid Partial Laryngectomy: Oncological and Functional Outcomes.

Introduction In the present study, we have reviewed the outcomes of patients with supracricoid partial laryngectomy (SCPL) in our institution. Our results show that SCPL is a well-tolerated procedure with generally good functional outcomes for patients with advanced laryngeal cancer. Objective We analyzed the oncological and functional results of a cohort of 35 patients who had undergone SCPL, and we highlighted the complications, identified the overall and disease-free survivals, demonstrating that the reconstructive laryngectomy guarantees the oncological safety and reproducibility of the oncological results, preserving the laryngeal functions and promoting an improvement in the patient's quality of life, favoring communication and interpersonal relationships. Methods Between 2010 and 2018, 35 patients underwent SCPL for primary and recurrent laryngeal squamous cell carcinomas, and they were divided into two subgroups: in 16 cases, the cricohyoidoepiglottopexy according to the Mayer-Piquet technique was performed, while the remaining 19 cases were submitted to the cricohyoidopexy according to the Labayle technique. In addition to evaluating the oncological results of patients undergoing reconstructive laryngectomy, the present study also aimed to evaluate the functionality of the residual larynx and the quality of life. Results The overall and disease-free survivals were of 83% and 76.3% respectively. All patients were able to swallow. The nasogastric tube was removed after a mean period of 21.8 days (range: 14 to 28 days). The mean decannulation time was of 23.4 days after surgery (range: 15 to 36 days). Conclusion The curves for the overall and disease-free survivals show that SCPL can guarantee oncological safety comparable to that of total laryngectomies in diseases in the intermediate stage and in carefully-selected advanced stages.

Neuroendocrine carcinoma of the larynx with Lambert-Eaton myasthenic syndrome: a rare case report and literature review.

This current report describes a rare clinical case of neuroendocrine carcinoma (NEC) of the larynx with associated Lambert-Eaton myasthenic paraneoplastic syndrome (LEMS). A 68-year-old male patient presented with severe dysphonia and dysphagia. He underwent a total laryngectomy and the excised lesion was extremely large. A pathological examination demonstrated 'morphological findings of a poorly differentiated carcinoma (G3) with aspects of neuroendocrine differentiation' (i.e. a poorly differentiated neuroendocrine carcinomas [PD-NEC]). Based on the patient's medical history and the immunohistochemical findings, he was treated with three cycles of neoadjuvant chemotherapy (cisplatin-etoposide) and then radiotherapy with a total dose of 70 Gy. Of the 10 cases of paraneoplastic syndrome (PNS) related to laryngeal NEC reported in the literature, nine of these syndromes were of an endocrine type. Only one case of PNS associated with laryngeal cancer had a neurological manifestation, which was LEMS. To the best of our knowledge, this current case has only one similar precedent in the literature and it is the second report of an association between a PD-NEC and LEMS. Laryngeal NECs are rare lesions with different prognostic characteristics. The diagnosis should be made using an endocrinological, neurological, radiological and histological multidisciplinary approach. A radical surgical approach is recommended.

Focus on localized laryngeal amyloidosis: management of five cases.

Amyloidosis is a group of idiopathic clinical syndromes caused by the deposition of insoluble fibrillar proteins (amyloid) in the extracellular matrix of organs and tissues. These deposits disrupt the function of the target organ. Amyloidosis can manifest as a systemic disease or a single-organ involvement (local form). Its etiology still remains unclear. Deposits of amyloid in the larynx are rare, accounting for between 0.2 and 1.2% of benign tumors of the larynx. In this retrospective study, we report the clinical aspects, diagnosis, treatment and follow-up of five female patients with localized laryngeal amyloidosis without systemic involvement. The patients were all treated successfully using microlaryngoscopy with CO2 laser or cold instruments. Prognosis is excellent; however, appropriate follow-up is an important part of the long-term management of this disease in order to prevent and control the possibility of local recurrence.

Validation of PROMIS Physical Function Instruments in Patients With an Orthopaedic Trauma to a Lower Extremity.

OBJECTIVES: To evaluate the reliability, convergent validity, known-groups validity, and responsiveness of the Patient-Reported Outcomes Measurement Information System (PROMIS) Mobility Computer Adaptive Test (CAT) and PROMIS Physical Function 8a Short Form. DESIGN: Prospective cohort study. SETTING: Two Level-I trauma centers. PATIENTS: Eligible adults with an isolated lower extremity trauma injury receiving treatment were approached consecutively (n = 402 consented at time 1, median = 80 days after treatment). After 6 months, 122 (30.3%) completed another assessment. INTERVENTION: Cross-sectional and longitudinal monitoring of patients. MAIN OUTCOME MEASUREMENTS: Floor and ceiling effects, reliability (marginal reliability and Cronbach's alpha), convergent validity, known-groups discriminant validity (weight-bearing status and fracture severity), and responsiveness (Cohen's d effect size) were evaluated for the PROMIS Mobility CAT, PROMIS Physical Function 8a Short Form, and 5 other measures of physical function. RESULTS: PROMIS PFSF8a and Foot and Ankle Ability Measure Activities of Daily Living Index had ceiling effects. Both PROMIS measures demonstrated excellent internal consistency reliability (mean marginal reliability 0.94 and 0.96; Cronbach's alpha = 0.96). Convergent validity was supported by high correlations with other measures of physical function (r = 0.70-0.87). Known-groups validity by weight-bearing status and fracture severity was supported as was responsiveness (Mobility CAT effect size = 0.81; Physical Function Short Form 8a = 0.88). CONCLUSIONS: The PROMIS Mobility CAT and Physical Function 8a Short Form demonstrated reliability, convergent and known-groups discriminant validity, and responsiveness in a sample of patients with a lower extremity orthopaedic trauma injury.

Preparedness and Mutuality Affect Quality of Life for Patients With Mechanical Circulatory Support and Their Caregivers.

BACKGROUND: Clinical and US regulatory guidelines for patients with mechanical circulatory support (MCS) require the identification of a caregiver to assist with MCS care. There is limited understanding of the impact of MCS caregiving on patients and caregivers. The purpose of this study was to examine how living with MCS affects the quality of life (QoL) of patients and their caregivers through the lens of preparedness and mutuality. METHODS AND RESULTS: The sample included 30 MCS patients and their caregivers. Semistructured qualitative interviews about factors contributing to QoL were conducted with patients and caregivers and analyzed using a 2-phase thematic process. Caregiving impacts QoL of MCS patients and their caregivers long term. When there was limited time to engage in decision-making about MCS implantation, people entered MCS caregiving relationships naive to its full demands. Although most people adjusted to the task demands, MCS caregiving had a significant impact, both positive and negative, on interpersonal relationships. We applied the concepts of preparedness and mutuality to help frame the understanding of the emergent themes of forced choice, adjustment, gratitude, relationship change, strain, and burden (both caregiver and patient perceived). Availability of networks of support was identified as a crucial resource. CONCLUSIONS: MCS caregiving affects QoL for both patients and caregivers. Specifically, preparedness, mutuality, and availability of supportive networks influence QoL of MCS patients and their caregivers. Caregiving relationships change over time. Long-term support by the MCS clinical team can help ensure that physical and emotional needs of MCS patients and caregivers are identified and addressed.

Longitudinal Validation of the PROMIS Physical Function Item Bank in Upper Extremity Trauma.

OBJECTIVES: To evaluate the reliability, validity, and responsiveness of the Patient-Reported Outcomes Measurement Information System (PROMIS) Upper Extremity Computer Adaptive Test (UE-CAT) and the 8-item Physical Function short form (PF-SF8a) for monitoring outcomes after musculoskeletal injuries in upper extremity trauma patients. DESIGN: Prospective cohort study. SETTING: Two Level-I trauma centers. PATIENTS: Eligible consecutive patients were approached and 424 consented at time 1 (median 9.7 weeks posttreatment). After 6 months, 132 patients (43% of the 307 eligible) completed follow-up measures. INTERVENTION: Cross-sectional and longitudinal monitoring of upper extremity trauma patients treated with or without surgery. MAIN OUTCOME MEASUREMENTS: Reliability, validity, and responsiveness of the UE-CAT and PF-SF8a. Internal consistency reliability, convergent validity correlations, and discriminant validity (by fracture severity and dominant/nondominant extremity groups) were calculated for PROMIS and non-PROMIS forms. Floor and ceiling effects were also examined at both assessment occasions. Responsiveness was evaluated using random-intercept mixed effects models and effect sizes. RESULTS: PROMIS measures had excellent reliability, correlated well with legacy measures, and were responsive to treatment. CONCLUSIONS: PROMIS measures had good statistical properties. In addition to the known advantages of PROMIS, such as lower patient burden and the ability to assess the broadest range of functioning, our data demonstrated that for patients with upper extremity limitations, a region-specific measure such as the UE-CAT may perform more favorably than an overall/full body physical function measure.

Validating Neuro-QoL short forms and targeted scales with people who have multiple sclerosis.

BACKGROUND: Multiple sclerosis (MS) is a chronic, progressive, and disabling disease of the central nervous system with dramatic variations in the combination and severity of symptoms it can produce. The lack of reliable disease-specific health-related quality of life (HRQL) measures for use in clinical trials prompted the development of the Neurology Quality of Life (Neuro-QOL) instrument, which includes 13 scales that assess physical, emotional, cognitive, and social domains, for use in a variety of neurological illnesses. OBJECTIVE: The objective of this research paper is to conduct an initial assessment of the reliability and validation of the Neuro-QOL short forms (SFs) in MS. METHODS: We assessed reliability, concurrent validity, known groups validity, and responsiveness between cross-sectional and longitudinal data in 161 recruited MS patients. RESULTS: Internal consistency was high for all measures (α = 0.81-0.95) and ICCs were within the acceptable range (0.76-0.91); concurrent and known groups validity were highest with the Global HRQL question. Longitudinal assessment was limited by the lack of disease progression in the group. CONCLUSIONS: The Neuro-QOL SFs demonstrate good internal consistency, test-re-test reliability, and concurrent and known groups validity in this MS population, supporting the validity of Neuro-QOL in adults with MS.

Health-related quality of life in mechanical circulatory support: Development of a new conceptual model and items for self-administration.

BACKGROUND: Generic and heart failure (HF)-specific health-related quality of life (HRQOL) instruments do not address unique burdens of mechanical circulatory support (MCS). This report describes (1) a conceptual model of adjustment to MCS and HRQOL, (2) the development of a new set of items to assess adjustment and HRQOL, and (3) establishes content validity of the new model and items. METHODS: We interviewed 15 expert clinicians, 16 patients with advanced HF, and 48 MCS patients. A grounded theory approach was used to systemically examine qualitative data. We developed a coding dictionary, with codes organized under concepts. A conceptual model of adjustment to MCS and HRQOL was developed. A set of relevant items was generated from the codes, concepts, and conceptual model. After items were refined, MCS patients participated in cognitive interviews to provide feedback on their relevance and acceptability. RESULTS: Patients described how having HF and MCS affected their daily lives. Three concepts regarding adjustment to MCS and its relationship to HRQOL emerged: (1) effect of disease and treatment (satisfaction with treatment, symptoms, and self-efficacy regarding self-care), (2) resources, and (3) implant strategy. From our codes, concepts, and model, we developed a set of 652 items that were categorized by concept. The item set was reduced from 652 items to 236 (36%), and 120 of these 236 items (51%) underwent cognitive debriefing. Our final set includes 239 items with evidence of content validity. CONCLUSIONS: Our newly developed model on adjustment to MCS and HRQOL and items will undergo further testing in the future.

Validity of the Neurology Quality-of-Life (Neuro-QoL) measurement system in adult epilepsy.

Epilepsy is a chronic neurological disorder that results in recurring seizures and can have a significant adverse effect on health-related quality of life (HRQL). The Neuro-QoL measurement initiative is an NINDS-funded system of patient-reported outcome measures for neurology clinical research, which was designed to provide a precise and standardized way to measure HRQL in epilepsy and other neurological disorders. Using mixed-method and item response theory-based approaches, we developed generic item banks and targeted scales for adults and children with major neurological disorders. This paper provides empirical results from a clinical validation study with a sample of adults diagnosed with epilepsy. One hundred twenty-one people diagnosed with epilepsy participated, the majority of which were male (62%) and Caucasian (95%), with a mean age of 47.3 (SD=16.9). Baseline assessments included Neuro-QoL short forms and general and external validity measures. The Neuro-QoL short forms that are not typically found in other epilepsy-specific HRQL instruments include Stigma, Sleep Disturbance, Emotional and Behavioral Dyscontrol, and Positive Affect and Well-Being. Neurology Quality-of-Life short forms demonstrated adequate reliability (internal consistency range=.86-.96; test-retest range=.57-.89). Pearson correlations (p<.01) between Neuro-QoL forms of emotional distress (anxiety, depression, stigma) and the QOLIE-31 Emotional Well-Being subscale were in the moderate-to-strong range (r's=.66, .71 and .53, respectively), as were relations with the PROMIS Global Mental Health subscale (r's=.59, .74 and .52, respectively). Moderate correlations were observed between Neuro-QoL Social Role Performance and Satisfaction and the QOLIE-31 Social Function (r's=.58 and .52, respectively). In measuring aspects of physical function, the Neuro-QoL Mobility and Upper Extremity forms demonstrated moderate associations with the PROMIS Global Physical Function subscale (r's=.60 and .61, respectively). Neuro-QoL measures of perceived cognitive function (executive function and general concerns) produced moderate-to-strong correlations with the QOLIE-31 Cognition subscale (r's=.65 and .75, respectively) and moderate relations with the Liverpool Adverse Events Profile (r's=.51 and .69, respectively). Finally, the Neuro-QoL Fatigue measure demonstrated moderate associations with the QOLIE-31 Energy/Fatigue subscale (r=-.65), Liverpool Adverse Events Profile (r=.69), and the Liverpool Seizure Severity Scale (r=.50). Five Neuro-QoL short forms demonstrated statistically significant responsiveness to change at 5-7months, including Fatigue, Sleep Disturbance, Depression, Positive Affect and Well-Being, and Emotional and Behavioral Dyscontrol. Overall, Neuro-QoL instruments showed good evidence for internal consistency, test-retest reliability, convergent validity, and responsiveness to change over several months. These results support the validity of Neuro-QoL to measure HRQL in adults with epilepsy.